Chiari Malformation

Chiari (pronounced: key-AR-ee) malformations are a group of conditions that result when a portion of the cerebellum (back part of the brain) grows outside of the skull and into the spinal canal. At Nemours Children’s Health System, our pediatric neuroscience teams are experts at recognizing the different signs and symptoms of Chiari malformations in children and providing effective, appropriate care. In fact, our pediatric neurosurgeons, neurologists and neuroradiologists are leading the way in developing best practice protocols and technologies — both medical and surgical options — to successfully diagnose and treat children with Chiari malformations.

Read More About Chiari Malformations in Children

A Chiari malformation is a general term for a group of conditions that result when the skull at the back of the head is too small or malformed and crowds the cerebellum (the back portion of the brain). When this happens, there is increased pressure on the brain and parts of the cerebellum (and sometimes the brainstem and other structures) extend or protrude downward through a hole in the skull (called the "foramen magnum") and typically into the spinal canal (the upper part of the spinal cord).

Types and Causes of Chiari Malformations

Chiari malformations can be congenital (present at birth) or acquired (occur over time). There are different types of Chiari malformations, from Type I (a milder form that may not require treatment) to Type IV (a very rare and severe congenital form caused by a malformed cerebellum).

There is no one cause of Chiari malformations, however it’s believed the condition is tied to problems during fetal development. These problems may occur due to genetic mutations (or changes) while the baby is still in the womb, congenital medical conditions (like spina bifida), nutritional deficiencies and other causes that are currently being researched.

Symptoms and Treating Chiari Malformations in Children

Chiari malformations symptoms can present at any age, and in milder forms (like Type I Chiari malformation), there may be no symptoms at all. In some cases, symptoms may begin in late childhood to early adulthood. Symptoms of Chiari malformations can include:

  • occipital headaches (at the back of the brain) that may worsen upon straining, coughing/sneezing or physical activity
  • dizziness and/or vertigo (balance problems)
  • vision problems (blurriness or involuntary eye movements)
  • problems swallowing
  • respiratory problems (like sleep apnea/obstructed breathing)
  • muscle weakness
  • tingling and/or numbness in the hands or feet
  • impaired fine motor skills (cutting, writing, tying shoes)
  • tinnitus (ringing in the ears)
  • chronic fatigue
  • loss of bowel or bladder function
  • spasticity (rigid or tight muscles)

Treating Chiari malformation involves alleviating the symptoms it causes and if appropriate, surgically repairing the malformation to reduce the pressure on the brain. Neurosurgery is not always necessary, particularly when there are no symptoms present. When surgical treatment (like posterior fossa decompression) is required, most children with less severe types do very well and grow up being active kids and independent adults.

Why Families Choose Nemours

Families choose Nemours because we’re wholly dedicated to building strong partnerships and providing family-centered care. Our team might know your child’s condition, but only you know your child best. We want you to be an active participant in the decision-making process, which is why we take the time to explain the diagnosis and treatment in understandable terms, present all of the options and invite your input to create the right treatment plan for your child.

We always take time listen to your concerns and provide personal service and comprehensive care for your child — and family — through emotional support, education and open communication.

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